Martijn. G. Slieker

Background: Right ventricular (RV) strain using two-dimensional speckle tracking is a reliable and clinically significant tool for detecting RV systolic dysfunction, but it varies by age, vendor, and software. Objectives: To establish pediatric age-specific normal values and Z-score equations for biventricular strain using GE Healthcare equipment and software. Methods: Children 0–18 years with structurally and functionally normal hearts, who visited the Wilhelmina Children’s Hospital Utrecht between October 2020 and December 2023, were retrospectively included and divided into age groups: 0 years, 1–4 years, 5–9 years, 10–13 years, and 14–18 years. Left ventricular (LV) and RV global longitudinal strain (GLS) and RV free wall longitudinal strain (FWGLS) were analyzed. Results: We included 129 subjects (57% male) (0 years: n = 17; 1–4 years: n = 22; 5–9 years: n = 34; 10–13 years: n = 35; 14–18 years: n = 20). Low R² values were strain-adjusted for age, height, and body surface area (all < 0.3), and the sample size limited Z-score equation reliability. Therefore, data are presented as mean ± SD or median [IQR] stratified by age. LV GLS, RV GLS, and RV FWGLS showed a nonlinear relationship with age, peaking at the 1–4 years age group and decreasing with age. Conclusions: LV GLS, RV GLS, and RV FWGLS showed age-related differences in children using GE equipment and software, which highlights the importance of age-specific normal strain values, including Z-score equations as a function of age.

The Pediatric Quality of Life Inventory™ (PedsQL™) family impact module is a widely used tool for assessing the health-related quality of life (HRQoL) of caregivers of children with chronic illnesses; however, it has not been available in Swahili. This study aimed to translate, culturally adapt, and validate the Swahili version of the PedsQL family impact module (FIM) among caregivers of children with heart disease in Tanzania. As a secondary aim, the study explored differences in caregiver HRQoL between those whose children had undergone surgery and those who had not, using the newly translated tool. A cross-sectional survey was conducted among 204 primary caregivers at a national cardiac referral centre. Standardised translation and adaptation procedures were followed. Psychometric evaluation included internal consistency reliability, construct validity through known-groups comparisons, and effect size estimation. The Swahili version demonstrated excellent internal consistency (Cronbach’s α = 0.99) and good construct validity. Socioeconomic status was the only consistent predictor of HRQoL in multivariable models. Caregivers in the operated group consistently reported higher HRQoL scores, highlighting the potential benefit of surgical intervention on family well-being. These findings strongly support the Swahili PedsQL FIM as a culturally appropriate and psychometrically sound tool. It offers promise for clinical care and future outcome evaluations in similar settings.

Background: Although genetic variants in MYH7 are the most frequent cause of pediatric genetic dilated cardiomyopathy (DCM), there are no studies available describing this entity. We sought to describe clinical features, analyze variant location, and explore predictors of bad prognosis in pediatric MYH7 ‐related DCM. Methods and Results: We evaluated clinical records from 44 patients (24 men; median age at diagnosis, 0.54 [interquartile range, 0.01–10.8] years) with pathogenic/likely pathogenic variants in MYH7 diagnosed with DCM at pediatric age (P =0.002) and LV ejection fraction ≤35% (HR, 4.00 [95% CI, 1.11–14.4]; P =0.03) were the best predictors of bad prognosis. Conclusions: Pediatric MYH7 ‐related DCM is characterized by early onset, frequent LV hypertrabeculation, and poor prognosis. Advanced New York Heart Association class and low LV ejection fraction emerged as predictors of end‐stage heart failure.

Tetralogy of Fallot (TOF) is typically treated in infancy but often done late in many resource-limited countries, jeopardizing surgical outcomes. This study examined the early results of children undergoing primary complete TOF repair at the Jakaya Kikwete Cardiac Institute (JKCI) in Tanzania, an emerging cardiac center in Eastern Africa. A retrospective cohort study of children ≤ 18 years undergoing primary TOF complete repair between 2019 and 2021 was conducted. Patients with complex TOF and those with obvious genetic syndrome were excluded. Data on socio-demography, pre-and postoperative cardiac complications, Intensive Care Unit (ICU) and hospital stay, and in-hospital and 30-day mortality were analyzed. Logistic regressions were employed to find the factors for mortality, ICU, and hospital stays. The I02 children underwent primary TOF complete repair were majority male (65.7%; n = 67), with a median age of 3.0 years (IQR: 2–6), ranging from 3 months to 17 years.Only 20 patients (19.6%) were below one year of age. Almost all (90%; n = 92) were underweight, with a mean BMI of 14.6 + 3.1 kg/m2 Haematocrits were high, with a median of 48.7 (IQR: 37.4–59.0). The median oxygen saturation was 81% (IQR:72–93). Over a third of patients (38.2%; n = 39) needed Trans annular patch (TAP) during surgery. The median ICU stay was 72 h (IQR:48–120), with ICU duration exceeding three days for most patients. The median hospital stay was 8.5 days (IQR:7–11), with 70 patients (68.2%)experiencing an extended hospital stay of > 7 days. Bacterial sepsis was more common than surgical site infection (5.6%; n = 6 vs. 0.9%;n = 1). No patient needed re-operation for the period of follow up. The in-hospital mortality rate was 5.9%, with no deaths occurring in children less than one year of age nor after discharge during the 30-day follow-up period. No statistically significant differences were observed in outcomes in relation to age, sex, levels of hematocrit and saturations, presence of medical illnesses, and placement of TAP. TOF repairs in this African setting at a national cardiac referral hospital face challenges associated with patients’ older age and compromised nutritional status during the surgery. Perioperative mortality rates and morbidity for patients operated at an older age remain elevated. It’s important to address these issues to improve outcomes in these settings. This study examined the clinical characteristics and 30-day surgical outcomes of patients with Tetralogy of Fallot who were treated in a low-middle-income setting.

Global Heart is the official and primary publication of the World Heart Federation, offering a platform for the dissemination of knowledge on research, developments, trends, solutions and public health programmes in the area of cardiovascular disease. Global Heart welcomes research results, points of view and educational material on the prevention, treatment and control of cardiovascular disease with a special focus on low and middle-income countries which are facing the brunt of epidemiological transition.Global Heart strongly encourages authors to adhere to CONSORT, STROBE, STARD, and PRISMA guidelines for reporting of clinical trials, observational studies, diagnostic test accuracy papers, and systematic reviews or meta-analyses. Authors are required for submission to download and complete the appropriate Equator Network checklist: http://www.equator-network.org/.

Background: Newborns with a critical congenital heart disease left undiagnosed and untreated have a substantial risk for serious complications and subsequent failure to thrive. Prenatal ultrasound screening is not widely available, nor is postnatal echocardiography. Physical examination is the standard for postnatal screening. Pulse oximetry has been proposed in numerous studies as an alternative screening method. This systematic review and meta-analysis aims to determine the diagnostic accuracies of both screening methods separately and combined. Methods: A systematic literature search of the Embase, PubMed, and Global Health databases up to 30 November 2023 was conducted with the following keywords: critical congenital heart disease, physical examination, clinical scores, pulse oximetry, and echocardiography. The search included all studies conducted in the newborn period using both physical examination and pulse oximetry as screening methods and excluded newborns admitted to the intensive care unit. All studies were assessed for risk of bias and applicability concerns using the QUADAS-2 score. The review adhered to the PRISMA 2020 statement guideline. Results: Out of 2711 articles, 20 articles were selected as eligible for meta-analysis. Cumulatively, the sample included 872,549 screened newborns. The pooled sensitivity of the physical examination screening method was found to be 0.69 (0.66–0.73 (95% CI)) and specificity was found to be 0.98 (0.98–0.98). For the pulse oximetry screening method, the pooled sensitivity and specificity yielded 0.78 (0.75–0.82) and 0.99 (0.99–0.99), respectively. The combined method of screening yielded improved diagnostic characteristics at a sensitivity and specificity of 0.93 (0.91–0.95) and 0.98 (0.98–0.98, respectively. Conclusions: The evidence indicates that combining both physical examination and pulse oximetry to screen for critical congenital heart disease exceeds the accuracy of either separate method. The main limitation is that solely newborns with suspected critical congenital heart disease were subjected to the reference standard. We recommend adapting both methods to screen for critical congenital heart diseases, especially in settings lacking standard fetal ultrasound screening. To increase the sensitivity further, we recommend increasing the screening time window and employing the peripheral perfusion index.

In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6–18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N > 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and “simple” lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VO₂peak, 37.3 ml/min/kg (25th–75th percentile 31.3–43.8)) varied between specific defects; patients with univentricular hearts had lower VO_2peak compared with other CHD. All groups had lower VO_2peak compared to healthy Dutch children. Males had higher VO_2peak, W_peak and O₂pulse_peak than females. Sex- and disease-specific distribution graphs for VO_2peak, W_peak and O2pulse_peak showed increase in variation with increase in height. Conclusion: Disease-specific distribution graphs for exercise capacity in children with CHD from a large multicentre cohort demonstrated varying degrees of reduced VO_2peak and W_peak. The distribution graphs can be used in the structured follow-up of patients with CHD to predict outcome and identify patients at risk. What is Known:• Children with congenital heart disease (COnHD) are at risk to develop heart failure, arrhytmia’s and other complications. Exercise capacity may be an important predictor for outcome in children with ConHD. In children, the interpretation of exercise capacity poses an additional challenge related to physical changes during growth. What is New:• In this report of a multi-center cohort >1300 childrewn with ConHD, we related the changes in exercise capacity to length. We demonstrated that exercise capacity was reduced as compared with healthy children and we observed variation between disease groups. Patients with a univentricular circulation (Fontan) had worse exercise capacity. We constructed disease specific charts of development of exercise capacity throughout childhood, accessible via a web-site. These graphs may help practitioner to guide children with ConHD.